Bilateral Vestibulopathy in Superficial Siderosis

Abstract

Background: Superficial siderosis (SS) is a rare condition in which hemosiderin, an iron storage complex, is deposited in neural tissues because of recurrent subarachnoid bleeding. Hemosiderin deposition in the vestibulocochlear nerve (CN VIII), brain, spinal cord and peripheral nerve can cause sensorineural hearing loss (SNHL) and postural imbalance, but much remains unknown about the vestibular manifestations of SS.Objectives: To report the clinical course, cochleovestibular status, and patterns of vestibulopathy during follow-up of a relatively large case series, and to discuss the possible pathophysiological mechanism of vestibular deterioration.Methods: Six patients diagnosed with SS by magnetic resonance imaging (MRI) were enrolled. Their medical records and radiological findings were retrospectively reviewed, particularly in terms of progression of the vestibulocochlear manifestations and the radiological characteristics.Results: All six patients had SNHL. Five of them exhibited progressive hearing loss over years, which was asymmetric in four. On their most recent evaluations, patients showed cerebellar ataxia with combined central and peripheral vestibulopathy on both sides (n = 4), a bilateral peripheral vestibulopathy (n = 1) or isolated central vestibulopathy (n = 1). Notably, the former four patients showed an evolution of isolated central vestibulopathy into combined central and peripheral vestibulopathy. Hypo-intense lesions on T2 weighted MRIs were evident around the cerebellum in all patients, but such lesions were observed around the brainstem in five and the CN VIII in four. The cochlea-vestibular dysfunction generally progressed asymmetrically, but no left-right asymmetry was evident on MRI.Conclusions: SS typically presents as bilaterally asymmetric, progressive cochleovestibular dysfunction with cerebellar ataxia. The pattern of vestibular dysfunction is usually combined central and peripheral vestibulopathy on both sides. Thus, precise identification of audiovestibular dysfunction and central signs is essential in SS, and patients with SS should undergo regular, comprehensive neurotological evaluation to optimize their treatments and prognosis.

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

“…This extremely rare disease is characterized by hemosiderin deposits particularly in glial cells of the CNS due to recurrent subarachnoid hemorrhage. Overall, only 30 case reports describing vestibular involvement in superficial siderosis are available in the medical literature so far 349 . Beside progressive bilateral audiovestibular dysfunction, patients often display cerebellar symptoms and other neurological deficits 350 351 .…”

“…Insgesamt ist bisher nur bei ca. 30 Patienten in der Literatur eine vestibuläre Beteiligung bei superfizieller Siderose beschrieben 349 . Neben einem progressiven bilateralen audiovestibulären Funktionsverlust finden sich häufig ein zerebelläres Syndrom und andere neurologische Ausfälle 350 351 .…”

Bilateral Vestibular Dysfunction

“…3). 57 Even though hearing is mostly preserved in patients with CABV or CANVAS, 43,44 hearing impairment is almost always present in superficial siderosis. Otherwise, findings of combined peripheral (positive HITs in both directions) and central (GEN) vestibulopathy may be observed in pregabalin intoxication.…”

Recent Advances in the Diagnosis of Audio-vestibular DisordersRunning head-Advances in the Diagnosis of Audio-vestibular Disorders

“…There are positive MRI findings possibly responsible for clinical manifestations in 232 patients (in 18.37% of the cases) as follows: in 86 patients (in 20.05%) with hearing loss, in 21 (in 21.88%) -with facial palsy, in 62 (in 18.18%) -with tinnitus, and in 63 (in 15.87% of the cases) -with vertigo. In six patients with superficial siderosis diagnosed by MRI, it deals with a sensorineural hearing loss, cerebellar ataxia with combined central and peripheral vestibulopathy on both sides (in four), as well as a bilateral peripheral vestibulopathy or isolated central vestibulopathy (in one patient each) (13). There is progressive hearing loss over year in five patients which is asymmetric in four of them.…”